Epidemiology, Presentation, and Evaluation

Neuroblastoma is the most common extracranial solid tumor of childhood and the most common malignancy of infancy. Primary tumors most commonly arise in the adrenal gland or along the sympathetic ganglion chain in the chest, abdomen, or pelvis. Presenting symptoms generally correspond to the site of the primary tumor, though patients with metastatic disease may present with constitutional symptoms as well. Common sites of metastatic disease include lymph nodes, bone marrow, and cortical bone. Treatment for children with neuroblastoma is determined based on risk for disease recurrence, as assessed by evaluation of clinical and tumor biologic factors. Current factors used in risk assessment include clinical stage, patient age at diagnosis, tumor histology, tumor MYCN amplification status, tumor segmental chromosomal aberration status, and tumor cell ploidy. Response to therapy is assessed using the revised International Neuroblastoma Response Criteria.